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Progress in Human Auditory and Vestibular Histopathology

edited by: S. Iurato & J.E. Veldman & G.R. Bock & M.J. Gleeson

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Publication details: Book. 1997. xii and 196 pages. Publication date: 1997-01-31. 145 figures and 19 tables. Hardbound.

ISBN: 978-90-6299-151-8 (ISBN 10: 90-6299-151-3; Kugler Publications)



The present symposium was held in November 1995 at the CIBA Foundation in London under the title The Current Status of Human Auditory and Vestibular Histopathology, and it brought back vivid memories of a similar symposium held at the same location that I attended in 1966. There have been many dramatic developments in electron microscopy over the ensuing 30 years, followed by the more recent development of immuno-histochernical and immunocytochemical methods and of molecular biology, which have all greatly contributed to our knowledge of the structure, ultrastructure and, indeed, the function of the inner ear.
The organizers of this symposium have gathered together the elite in the field of clinical and laboratory research, and, as a pathologist, I could not help but admire the superiority of clinical researchers in having been aware that "pathologists have always fought shy of the apparently barren regions of the temporal bone." The program could, arbitrarily, be divided into several sections. There were many instructive papers on some of the important normal features of the cells of the middle and inner ear, beginning with the beautifully illustrated talk by Burkhard Hussl on the dendritic cells of the tympanic membrane. Hussl also reviewed the origin and function of the Langerhans' T-cell microenvironment in the lining of epidermoid cholesteatoma. Electron microscopy, as presented by Joseph Nadol, covered not only the normal but also some of the pathological problems, in which it continues to be of valuable assistance to both the diagnostician as well as the research worker. This was clearly shown by Heidi Felix, a worthy pupil of the late and sadly missed Heinrich Spoendlin, in her painstaking analysis of the human cochlear neurons; as well as by Michael Gleeson in his fundamental study of the human peripheral vestibular system. Among other things, these studies confirmed that the large-diameter nerve fibers are absent from the vestibular nerve of elderly persons, thereby affecting the timing of nerve conduction and the sense of balance itself. Several purely pathological papers were also presented. One of these dealt with the clinical and histo-pathological picture of AIDS: an impressive, almost heroic, investigation carried out by Sava Soucek and Leslie Michaels. The latter described the inner ear in vestibular schwannomas, based on the collection of sections by the late Professor Hallpike, which were added to the collection of the Frolich Temporal Bone Laboratory at the Institute of Laryngology and Otology in London. The value of the delicate surface preparation technique for the inner ear in labyrinthine pathology was illustrated by Lars-Göran Johnsson in his contribution. Otosclerosis was the subject of two papers. Frank Decleau described his scanning electronmicroscopical findings in otosclerosis, and, using the polymerase chain reaction, Hans Niedermeyer provided further evidence of the viral cause of this disease, thereby enhancing our earlier immunohistochernical results. Immunohistochemistry in the study of the intermediate filament proteins forming the cytoskeleton of cells of the inner ear has been widely employed and reviewed by Anniko, Altermatt and Veldman, who give a state-of-the-art survey in their instructive reports. One recurring observation was the importance of adequate fixation, and Georg Kanonier described a difficult perilymphatic-perfusion technique, emphasizing, as others have done, the significance of the interval between death and tissue fixation of the human temporal bone. Klaus Qvortrup described an animal model as the basis for future submicroscopical studies of the inner ear. Other papers with an immunohistochemical timbre were presented by Anneliese Schrot-Fischer, Burkhard Hussl and David Lim. Mads Sørensen described the fundamental modelling and remodelling changes in the otic capsule. To complete this volume on Progress in Human Auditory and Vestibular Histopathology, we have added (by invitation) Harold Schuknecht's contribution on inner ear pathology in autoimmune disease.
Perhaps the most valuable feature of the meeting was the time allotted to the discussion of the presented papers, but even more importantly, to the discussion of more general problems: future programs, the collaboration between researchers and laboratories, and, last but not least, the effect of the dwindling number of autopsies which is leading to a dearth of suitable bone specimens. This was never anticipated by our teachers, Hallpike, Dohlmann, Ormerod and others during previous meetings on the biology and pathology of the inner ear.
As with earlier workshops held by the European working group, referred to by Salvatore Iurato, Göran Bredberg and Gregory Bock, these successful discussions cannot be expected to solve all our problems. Indeed, they may have posed more questions than they have answered, but, in the spirit of the Nobel Prize Laureate, Sir MacFarlane Burnet: "There can never be more than a more detailed and a more accurate approach towards the forever unattainable goal of full understanding."

I. Friedmann, MD, DSc, FRCS, FRC Path, DCP

Table of Contents

Table of Contents

List of Contributors

The European Working Group on Human Auditory and Vestibular Histopathology
Gregory R. Bock and Salvatore Iurato

An update on the NIDCD National Temporal Bone Hearing and Balance Pathology Resource Registry in the USA
Joseph B. Nadol, Jr.

Dendritic cells in the human tympanic membrane: an overview
Burkhard Hussl, Nikolaus Romani and Anneliese Schrott-Fischer

Tympanosclerosis of the human tympanic membrane
Burkhard Hussl and David J. Lim

Spatial organization of bone modelling and remodelling in the otic capsule
Mads S. Sørensen and Poul Bretlau

Scanning electron microscopy of normal and otosclerotic bone in the region of the oval window
Frank F.J Declau, Dietrich W. Scheuermann, Thomas Somers and Paul Van de Heyning

Otosclerosis and measles virus: immunological and molecular results
Hans P. Niedermeyer and Wolfgang Arnold

Electron microscopy of the human ear: anatomy and pathology
Joseph B. Nadol, Jr.

Morphological correlates of the barrier sealing the intrastrial compartment
Liliana Luciano, Salvatore Iurato, Gebhard Reiss, Stephan Beck and Enrico Reale

Stria vascularis in aging human individuals
Margarete Hamm-Pauler, Edward Felder, Georg Kanonier and Anneliese Schrott-Fischer

Perilymphatic perfusion: influence of the postmortem period on the quality of tissue fixation
Georg Kanonier, Edward Felder, Arne Scholtz, Margarete Hamm-Pauler, Wei-Jia Kong and Anneliese Schrott-Fischer

The cochlear neurons in humans
Heidi Felix, Michael J. Gleeson, Anita Pollak and Lars-Göran Johnsson

Quantitative and ultrastructural studies on the human peripheral vestibular system
Michael J. Gleeson, Heidi Felix and Lars-Göran Johnsson

Ultrastructural analysis and reconstruction of human vestibular organs
Arne W. Scholtz, Edward Felder, Georg Kanonier, Karl Thurner and Anneliese Schrott-Fischer

Changes in the inner ear in vestibular schwannoma
Dinsuhaimi Sidek, Leslie Michaels and Anthony Wright

Labyrinthine pathology in a deaf patient with infantile onset of spinocerebellar ataxia (IOSCA)
Lars-Göran Johnsson, Heidi Felix, Anders Paetau and Tuula Lönnqvist

Inner ear pathology in autoimmune disease
Harold F. Schuknecht

Clinical and histopathological aspects of ear disease in the acquired immunodeficiency syndrome
Sava Soueek, Leslie Michaels and Jianning Liang

Immunohistochernical investigations on neurotransmitters in human temporal bones
Anneliese Schrott-Fischer, Arne W. Scholtz, Georg Kanonier, Margarete Hamm-Pauler and Wei-Jia Kong

Human temporal bone sections in immunomorphological research: current status
Matti Anniko, Masaya Takumida and Wolfgang Arnold

Immunohistochemistry of the human endolymphatic sac
Hans J. Altermatt and Wolfgang Arnold

Glyconjugates as components of the tectorial membrane of the human fetal cochlea
Angeles Ibáñez, Alicia Rodríquez?Ramos, Mariví Bartolomé, Francisco Valderrama,
Manuel Remezal, Carlos Micó, José Dargallo and Javier Salmean

Intermediate filament proteins in the membranous labyrinth: an animal model
Jan E. Veldman, Ippei Takagi and John C.M.J de Groot

The endolymphatic duct in the rat: an ultrastructural study
Klaus Qvortrup, J. Rostgaard and Poul Bretlau

Subject Index

Index of Authors

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